Raymond Syndrome

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Article initiated by:

Andrew Go Lee, MD, Evan Wotipka, MD, Melody Ziari, Nagham Al-Zubidi, MD, Noor Laylani, M.D., Pamela Davila-Siliezar, MD, Zachary Richards

All contributors:

Andrew Go Lee, MD, Bryan Garth Pearson, Evan Wotipka, MD, Melody Ziari, Geulah S Ben-David, Elizabeth Arogundade, Nagham Al-Zubidi, MD, Noor Laylani, M.D., Osama Al Deyabat, MBBS, Sanjana Jaiswal

Assigned editor:

Osama Al Deyabat, MBBS

Review:

Assigned status Up to Date

 by Osama Al Deyabat, MBBS on September 4, 2024.

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Disease Entity

Raymond syndrome consists of a collection of clinical findings due to an insult in the mid-pons. One study separates Raymond syndrome into two distinct types:

• 1. Classic Raymond syndrome, which is characteristically produced via a lesion involving the ipsilateral abducens fascicle and the undecussated corticofacial and corticospinal fibers.
• 2. Common Raymond syndrome, which is similar to the classic type, but differs in that it spares the corticofacial fibers.

Therefore, the clinical manifestations of classic Raymond syndrome, which is significantly less common than the common subtype, consist of an ipsilateral abducens nerve palsy (abducens fascicle insult), contralateral central facial paresis (corticofacial insult), and a contralateral hemiparesis (corticospinal insult).

History

Raymond syndrome was first described in 1895 by Fulgence Raymond. He described a 39-year-old woman with syphilis presenting with an abducens nerve palsy, contralateral facial paresis, and hemiparesis. He suggested that a lesion at the facial decussation in the medial caudal pons could result in these symptoms. Since that time, only a handful of cases of Raymond syndrome have been reported in the literature.

Etiology

Raymond syndrome is caused by a lesion located in the medial ventral caudal pons, which gives rise to the characteristic features. Determining the exact etiology is challenging with only a few reported cases, but small vessel disease appears to be the most likely cause given that most of the reports mention hypertension as a prominent risk factor. Raymond syndrome secondary to pontine infarction, hemorrhage from a cavernous hemangioma at the pontomedullary junction, and lacunar infarct has been documented in the literature.

Clinical Manifestations

Raymond syndrome is characterized by ipsilateral abducens nerve palsy, contralateral hemiparesis, and facial paralysis. However, pure Raymond syndrome is extremely rare as there are many nuclei and fibers near the root of the abducens nerve. There is a debate regarding facial involvement, due to the uncertain route of the corticofacial fibers in the brainstem. Lesions involving the corticofacial decussation at the level of the abducens nerve would result in contralateral facial paresis. By contrast, lesions in a more dorsal area would produce an isolated abducens nerve palsy. The fibers have multiple routes, including an aberrant bundle near the medial meniscus in the pontine tegmentum. Corticofacial fibers that travel dorsally would be unaffected by a lesion in the ventral pons, resulting in Raymond syndrome with facial sparing. In patients presenting with a sixth nerve palsy and contralateral hemiplegia, regardless of a central facial palsy, a lesion in the ventral pons should be suspected and Raymond syndrome should be considered.  

Diagnostic

There are no clearly defined diagnostic criteria for Raymond syndrome, so the diagnosis is made based on a combination of radiographic and clinical findings. Sixth nerve palsy and contralateral hemiparesis are necessary features while contralateral facial involvement is rare. Neuroimaging may demonstrate cytotoxic edema in the ventromedial mid-pons.

Management

The treatment of Raymond syndrome depends on the etiology. Most commonly Raymond syndrome, like other insults to the brainstem, is a result of vascular disease. Thus, the use of tPA (tissue-type plasminogen activator) can be utilized only if it is administered within 4-5 hours of symptom onset and if there are no other contraindications to therapy (recent hemorrhage, coagulation therapy, etc.). Because ipsilateral abducens nerve palsy is a signature symptom of Raymond syndrome, the use of prisms and surgical correction can help treat bothersome diplopia, especially if it has not resolved within several months of the incident. The prognosis for recovery is dependent on multiple factors, including baseline function, age of onset, and the ability of the patient to undergo physical therapy.